본문 바로가기
KERD
논문KCI 등재

초로기 치매 증상으로 발현된 후천면역결핍증에서의 진행다초점백질뇌병증 증례

A Case of Progressive Multifocal Leukoencephalopathy in Acquired Immune Deficiency Syndrome Initially Presented with Early Onset Dementia

2

조회

0

다운로드

0

인용

0

참고문헌

키워드

주제분류신경과학

초록

Progressive multifocal leukoencephalopathy (PML) is a very rare and often fatal demyelinating disease of central nervous system (CNS), which mostly occurs in patients with immunosuppression such as acquired immunodeficiency syndrome (AIDS) patients, transplant patients and patients receiving chemotherapy. PML usually manifests with acute or subacute neurologic deficit. and its late diagnosis may lead death or significant permanent disability. We report a 33-year old man diagnosed with PML in AIDS, who initially presented with gradual onset of dementia. Most symptoms of PML were progressed rapidly for several months, and characterized by focal neurological symptoms. On the other hand, we were experienced in patients without focal neurological symptoms and ongoing overall cognitive decline slowly. Patients with immunosuppression can be presented in a variety of neurological symptoms, detailed examinations for cognitive functions were needed in early stage of the disease.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a very rare and often fatal demyelinating disease of central nervous system (CNS), which mostly occurs in patients with immunosuppression such as acquired immunodeficiency syndrome (AIDS) patients, transplant patients and patients receiving chemotherapy. PML usually manifests with acute or subacute neurologic deficit. and its late diagnosis may lead death or significant permanent disability. We report a 33-year old man diagnosed with PML in AIDS, who initially presented with gradual onset of dementia. Most symptoms of PML were progressed rapidly for several months, and characterized by focal neurological symptoms. On the other hand, we were experienced in patients without focal neurological symptoms and ongoing overall cognitive decline slowly. Patients with immunosuppression can be presented in a variety of neurological symptoms, detailed examinations for cognitive functions were needed in early stage of the disease.

참고문헌 (12)

  1. Weissert R, "Progressive multifocal leukoencephalopathy", J Neuroimmunol, 231, pp.73~77 (2011)
  2. Berger JR, "Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy", Ann Neurol, 44, pp.341~349 (1998)
  3. Lee JH, "One case of progressive multifocal leukoencephalopathy complicating chronic myelocytic leukemia", J Korean Neurol Assoc, 14, pp.282~288 (1996)
  4. 박병사, "Progressive Multifocal Leucoencephalopathy Isolated to Posterior Fossa in a Patient with AIDS: DWI and 1H-MRS Features", 대한영상의학회지, 63(5), pp.403~407 (2010), 대한영상의학회
  5. 고훈영, "전신홍반루푸스 환자에서 발생한 진행성 다초점 백색질 뇌증 1예", 대한류마티스학회지, 15(2), pp.159~164 (2008), 대한류마티스학회
  6. 강현희, "중증근무력증과 흉선종 치료 중 발생한 진행다초점백질뇌병증", 대한신경과학회지, 30(4), pp.312~315 (2012), 대한신경과학회
  7. Tan CS, "Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis", Lancet Neurol, 9, pp.425~437 (2010)
  8. Berger JR, "Progressive Multifocal Leukoencephalopathy Associated with Human Immunodeficiency Virus Infection : a Review of the Literature with a Report of Sixteen Cases", Ann Intern Med, 107, pp.78~87 (1987)
  9. Horger M, "Common and uncommon imaging findings in progressive multifocal leukoencephalopathy(PML)with differential diagnostic considerations", Clin Neurol and Neurosurg, 114, pp.1123~1130 (2012)
  10. Naess H, "Progressive multifocal leucoencephalopathy in an immunocompetent patient with favourable outcome. A case report", BMC Neurol, 10, pp.32~ (2010)
  11. Lima MA, "Clinical outcome of long-term survivors of progressive multifocal leukoencephalopathy", J Neurol Neurosurg Psychiatry, 81, pp.1288~1291 (2010)
  12. Frederik Neess Engsig, "Incidence, Clinical Presentation, and Outcome of Progressive Multifocal Leukoencephalopathy in HIV-Infected Patients during the Highly Active Antiretroviral Therapy Era: a Nationwide Cohort Study", Infect Dis, 199, pp.77~83 (2009)

관련 논문AI추천

이 간행물의 다른 논문 (4건)